DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

E80 Disorders of porphyrin and bilirubin metabolism

  • Header · not billable

E80 is not billable. The category heads 10 billable codes in FY2027, grouped under 8 subdivisions.

Index → code

  • Takahara's disease → E80.3
  • Acatalasemia, acatalasia → E80.3
  • Porphyria (South African) → E80.20

Where it sits

  1. Chapter 4 Endocrine, nutritional and metabolic diseases (E00-E89)
  2. E70-E88 Metabolic disorders (E70-E88)
  3. E80 Disorders of porphyrin and bilirubin metabolism
Billable codes
10
Subdivisions
8
CC / MCC codes
6 / 0
HCC V28 codes
6
New FY2027
0
Index entries
41

Codes in E80

  1. E80.0
    Hereditary erythropoietic porphyria
    BillableCC50
    Inclusion terms 2

    Inclusion terms

    • Congenital erythropoietic porphyria
    • Erythropoietic protoporphyria
  2. E80.1
    Porphyria cutanea tarda
    BillableCC50
  3. E80.2
    Other and unspecified porphyria
    Header
  4. E80.20
    Unspecified porphyria
    BillableCC50
    Inclusion terms 1

    Inclusion terms

    • Porphyria NOS
  5. E80.21
    Acute intermittent (hepatic) porphyria
    BillableCC50
  6. E80.29
    Other porphyria
    BillableCC50
    Inclusion terms 1

    Inclusion terms

    • Hereditary coproporphyria
  7. E80.3
    Defects of catalase and peroxidase
    BillableCC50
    Inclusion terms 1

    Inclusion terms

    • Acatalasia [Takahara]
  8. E80.4
    Gilbert syndrome
    Billable
  9. E80.5
    Crigler-Najjar syndrome
    Billable
  10. E80.6
    Other disorders of bilirubin metabolism
    Billable
    Inclusion terms 2

    Inclusion terms

    • Dubin-Johnson syndrome
    • Rotor's syndrome
  11. E80.7
    Disorder of bilirubin metabolism, unspecified
    Billable

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

Sources of each column.

Instructions

At E80

Includes

  • defects of catalase and peroxidase

Notes that also apply

Block E70-E88 instructions (Excludes1 5, Excludes2 1): printed on the block page.

Chapter 4 instructions (Notes 1, Excludes1 1): printed on the chapter 4 page.

Index entries

  • Protoporphyria, erythropoietic → E80.0
  • Porphyria (South African) → E80.20
  • Coproporphyria, hereditary → E80.29
  • Acatalasemia, acatalasia → E80.3
  • Takahara's disease → E80.3
  • Bilirubinemia, familial nonhemolytic → E80.4
  • Gilbert's disease or syndrome → E80.4
  • Crigler-Najjar disease or syndrome → E80.5
  • Dubin-Johnson disease or syndrome → E80.6
  • Rotor's disease or syndrome → E80.6
  • Bilirubin metabolism disorder → E80.7
  • Porphyria (South African)erythropoietic (congenital) (hereditary) → E80.0
  • Porphyria (South African)cutanea tarda (hereditary) (symptomatic) → E80.1
  • Porphyria (South African)hepatocutaneous type → E80.1
  • Porphyria (South African)acquired → E80.20
  • Porphyria (South African)due to drugs → E80.20
  • Porphyria (South African)secondary → E80.20
  • Porphyria (South African)toxic NEC → E80.20
  • Porphyria (South African)variegata → E80.20
  • Porphyria (South African)acute intermittent (hepatic) (Swedish) → E80.21
  • Defect, defectivecatalase → E80.3
  • Defect, defectiveperoxidase → E80.3
  • Cholemiafamilial (simple) (congenital) → E80.4
  • CholemiaGilbert's → E80.4
  • Jaundice (yellow)familial nonhemolytic (congenital) (Gilbert) → E80.4
  • Jaundice (yellow)nonhemolytic congenital familial (Gilbert) → E80.4
  • Deficiency, deficientglucuronyl transferase → E80.5
  • Bilirubin metabolism disorderspecified NEC → E80.6
  • Defect, defectivebilirubin excretion NEC → E80.6
  • Disorder (of)bilirubin excretion → E80.6
  • Hyperbilirubinemiaconstitutional → E80.6
  • Hyperbilirubinemiafamilial conjugated → E80.6
  • Polyneuropathy (peripheral)in (due to)porphyria → E80.20 [G63]
  • Defect, defectiveenzymecatalase → E80.3
  • Defect, defectiveenzymeperoxidase → E80.3
  • Jaundice (yellow)familial nonhemolytic (congenital) (Gilbert)Crigler-Najjar → E80.5
  • Disease, diseasedmetabolic, metabolismbilirubin → E80.7
  • Disorder (of)metabolism NOSbilirubin → E80.7
  • Jaundice (yellow)newborndue to or associated withGilbert syndrome → E80.4
  • Jaundice (yellow)newborndue to or associated withCrigler-Najjar syndrome → E80.5

1 more, longer entries are not listed; the search box finds them by word.

NCHS Alphabetic Index. Index first, then the Tabular List.

Hierarchy